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Hypoventilation Syndromes

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Abstract

In patients with impaired inspiratory muscle function or altered respiratory system mechanics, an imbalance between load and capacity can arise. The ventilatory control system normally compensates for this by increasing drive to maintain adequate alveolar ventilation levels, thereby keeping arterial CO2 within its normal range. To reduce work of breathing, a pattern of reduced tidal volume and increased respiratory rate occurs. This pattern itself may eventually reduce effective ventilation by increasing dead space ventilation. However, the impact of sleep on breathing and its role in the development of diurnal respiratory failure is often overlooked in this process. Sleep not only reduces respiratory drive, but also diminishes chemoresponsiveness to hypoxia and hypercapnia creating an environment where significant alterations in oxygenation and CO2 can occur. Acute increases in CO2 load especially during rapid eye movement sleep can initiate the process of bicarbonate retention which further depresses ventilatory responsiveness to CO2. Treatment of hypoventilation needs to be directed toward factors underlying its development. Nocturnal noninvasive positive pressure therapy is the most widely used and reliable strategy currently available to manage hypoventilation syndromes. Although this may not consistently alter respiratory muscle strength or the mechanical properties of the respiratory system, it does appear to reset chemosensitivity by reducing bicarbonate, resulting in a more appropriate ventilatory response to CO2 during wakefulness. Not only is diurnal hypoventilation reduced with noninvasive ventilation, but quality of life, functional capacity and survival are also improved. However, close attention to how therapy is set up and used are key factors in achieving clinical benefits. © 2014 American Physiological Society. Compr Physiol 4:1639‐1676, 2014.

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Figure 1. Figure 1. Changes in minute ventilation (VI), tidal volume (VT) and respiratory rate from wakefulness to sleep in patients with nocturnal hypoventilation (Desaturators), patients with eucapnic OSA during effective CPAP, and healthy normal controls. In the nocturnal hypoventilation group, the larger relative falls in VT were not offset by changes in respiratory rate so that an overall reduction in VI occurred. [Adapted, with permission, from Becker et al. 1999; Breathing during sleep in patients with nocturnal desaturation. Am J Respir Crit Care Med, 159:112‐118. Reprinted with permission of the American Thoracic Society. Copyright © 2014 American Thoracic Society. Official Journal of the American Thoracic Society (.)]
Figure 2. Figure 2. Recording of respiratory muscle activity and chest wall movement from a patient with scoliosis during sleep. There is widespread recruitment of accessory respiratory muscles during NREM sleep. Note also the activation of abdominal muscle activity (EMGABDO) during expiration. With the onset of REM sleep, a generalized reduction in inspiratory muscle activity occurs, with complete loss of abdominal EMG activity. This produces a marked reduction in chest wall movement with reduced airflow. [Adapted, with permission, from Becker et al. 1999; Breathing during sleep in patients with nocturnal desaturation. Am J Respir Crit Care Med, 159:112‐118. Reprinted with permission of the American Thoracic Society. Copyright © 2014 American Thoracic Society. Official Journal of the American Thoracic Society (.)]
Figure 3. Figure 3. Resting breathing in an obese subject (BMI 42 kg/m2) when seated (left), supine without CPAP (middle) and with CPAP (right). The change in end‐expiratory esophageal baseline pressure is reflected by the horizontal dotted lines (nos 1‐3). There is PEEPi of around 6 cmH2O (vertical broken lines indicate the start of inspiratory flow, with the difference between horizontal line 2 and 4 indicating PEEPi). Zero flow is indicated by the horizontal line. In the right panel the patient is supine breathing with CPAP at 6 cmH2O. Neural respiratory drive to the diaphragm increases when changing posture from sitting to supine and reduces with CPAP; PEEPi is offset by CPAP and pressure swings of Poes and Pdi are smaller. CPAP, continuous positive airway pressure; EMGdi, electromyogram of the diaphragm; Poes, esophageal pressure; Pgas, gastric pressure; Pdi, transdiaphragmatic pressure (= Pgas‐Poes); PEEPi, intrinsic positive end expiratory pressure. [Reproduced, with permission, from Thorax, Steier et al. 64:719‐725, 2009 with permission from the BMJ Publishing Group Ltd (.)]
Figure 4. Figure 4. High upper airway resistance is present in OHS patients not only when supine but during sitting. Total respiratory resistance (Rrs) at different oscillometry frequencies measured in awake sitting (A: upper panel) and supine (B: lower panel) in normal controls without OSA (Group I), in patients with moderate‐severe OSA and BMI ≤ 35 kg/m2 (Group IIa) or BMI > 35 kg/m2 (Group IIb) and patients with OHS (Group III). In sitting, there was no significant difference in Rrs at any oscillometry frequency between normal subjects and those with eucapnic OSAS, although Rrs at all frequencies was higher in the eucapnic OSAS compared to normal controls when supine (#P < 0.05). In patients with OHS, significantly higher Rrs was seen at all oscillometry frequencies compared to the other three groups in both sitting and when supine (*P < 0.05). [Reprinted from Respir Physiol Neurobiol. Vol 139, Lin et al, Oral airway resistance during wakefulness in eucapnic and hypercapnic sleep apnea syndrome. pg 215‐224, 2004, with permission from Elsevier. (.)]
Figure 5. Figure 5. Conceptual illustration of the impact of the interapneic period on the accumulation of CO2 during sleep in patients with upper airway obstruction. The accumulation of CO2 during the hypopnea is offset during the interapnea period by sufficient ventilation over a sufficient period to eliminate the previous CO2 build up. However, if more CO2 is accumulated than can be excreted in the interapneic period, a small net rise in CO2 occurs. If this process is repeatedly frequently, a rise in PaCO2 will occur. [Reprinted with permission from Berger et al., 2002. J of Appl Physiol 93:917‐924) ().]
Figure 6. Figure 6. In patients with a neuromuscular and chest wall disorder, the development of nocturnal hypoventilation portends the development of awake hypercapnia within the next 12 to 24 months. These 10 patients represent a control group allocated to no NIV over a 2 year period. The light‐colored bar represents the period of time the subject remained NIV‐free. By 12 months, 70% of the group required NIV, and by 24 months only one subject did not fulfill the criteria to commence NIV. (Reproduced from Thorax, Ward et al. 60:1019‐1024, 2005 with permission from the BMJ Publishing Group Ltd) ().
Figure 7. Figure 7. Scatter plots (left‐hand panel) and receiver operator curves (right‐hand panel) for the predictive thresholds for Inspiratory vital capacity for the (A) onset of sleep‐disordered breathing (SDB), (B) continuous nocturnal hypoventilation and (C) diurnal respiratory failure. [Reproduced from Thorax, Ragette et al. 57:724‐728, 2002 () with permission from the BMJ Publishing Group Ltd.]
Figure 8. Figure 8. The “dose response” of NPPV showing the change in PaCO2 (left panel) and daytime sleepiness (ESS) (right panel) in patients with neuromuscular and restrictive thoracic disorders after 3 months of therapy. The dotted line represents the threshold below which NPPV does not have a consistent effect on PaCO2 or daytime sleepiness. Abbreviations: ESS—Epworth Sleepiness Scale; Use p.n.—use per night. [Reproduced from Thorax, Nickol et al. 60:754‐760, 2005 () with permission from the BMJ Publishing Group Ltd.]
Figure 9. Figure 9. Illustration of overventilation during the spontaneous mode of bilevel ventilation. Note the markedly reduced TcCO2 (right panel). Reducing CO2 below the apneic threshold will create central events which are associated with significant oxygen desaturation. Note the loss of diaphragm EMG activity along with absence of respiratory efforts on the thoracic and abdominal bands. This breathing abnormality occurs commonly at sleep onset or following arousal if excessive levels of pressure support are used in the spontaneous mode.
Figure 10. Figure 10. Examples of the more common asynchronies that can occur during NPPV therapy. Such events may not be apparent during wakefulness but can impact on the quality of ventilation during sleep. Panel A illustrates ineffective efforts caused by high leak. Small upward deflections can be seen in the airflow channel without a corresponding EPAP to IPAP transition on the pressure trace. Inspiratory efforts go unrewarded. Note also the generalized reduction in thoracic band movement during the leak period and a gradual fall in SpO2. The first, third, and fifth breaths on the airflow and pressure channels in Panel B show the machine has cycled twice with a very short expiratory period between the two during a single inspiratory effort (Thoracic and Abdominal bands). This can indicate pressurization is too short compared to inspiratory demand or the level of inspiratory support is too low (). Panel C illustrates autotriggering—a rapid succession of multiple machine pressurizations clearly above the patient's native respiratory rate (). This can occur if the inspiratory trigger has been set too low, especially in the presence of leak ().


Figure 1. Changes in minute ventilation (VI), tidal volume (VT) and respiratory rate from wakefulness to sleep in patients with nocturnal hypoventilation (Desaturators), patients with eucapnic OSA during effective CPAP, and healthy normal controls. In the nocturnal hypoventilation group, the larger relative falls in VT were not offset by changes in respiratory rate so that an overall reduction in VI occurred. [Adapted, with permission, from Becker et al. 1999; Breathing during sleep in patients with nocturnal desaturation. Am J Respir Crit Care Med, 159:112‐118. Reprinted with permission of the American Thoracic Society. Copyright © 2014 American Thoracic Society. Official Journal of the American Thoracic Society (.)]


Figure 2. Recording of respiratory muscle activity and chest wall movement from a patient with scoliosis during sleep. There is widespread recruitment of accessory respiratory muscles during NREM sleep. Note also the activation of abdominal muscle activity (EMGABDO) during expiration. With the onset of REM sleep, a generalized reduction in inspiratory muscle activity occurs, with complete loss of abdominal EMG activity. This produces a marked reduction in chest wall movement with reduced airflow. [Adapted, with permission, from Becker et al. 1999; Breathing during sleep in patients with nocturnal desaturation. Am J Respir Crit Care Med, 159:112‐118. Reprinted with permission of the American Thoracic Society. Copyright © 2014 American Thoracic Society. Official Journal of the American Thoracic Society (.)]


Figure 3. Resting breathing in an obese subject (BMI 42 kg/m2) when seated (left), supine without CPAP (middle) and with CPAP (right). The change in end‐expiratory esophageal baseline pressure is reflected by the horizontal dotted lines (nos 1‐3). There is PEEPi of around 6 cmH2O (vertical broken lines indicate the start of inspiratory flow, with the difference between horizontal line 2 and 4 indicating PEEPi). Zero flow is indicated by the horizontal line. In the right panel the patient is supine breathing with CPAP at 6 cmH2O. Neural respiratory drive to the diaphragm increases when changing posture from sitting to supine and reduces with CPAP; PEEPi is offset by CPAP and pressure swings of Poes and Pdi are smaller. CPAP, continuous positive airway pressure; EMGdi, electromyogram of the diaphragm; Poes, esophageal pressure; Pgas, gastric pressure; Pdi, transdiaphragmatic pressure (= Pgas‐Poes); PEEPi, intrinsic positive end expiratory pressure. [Reproduced, with permission, from Thorax, Steier et al. 64:719‐725, 2009 with permission from the BMJ Publishing Group Ltd (.)]


Figure 4. High upper airway resistance is present in OHS patients not only when supine but during sitting. Total respiratory resistance (Rrs) at different oscillometry frequencies measured in awake sitting (A: upper panel) and supine (B: lower panel) in normal controls without OSA (Group I), in patients with moderate‐severe OSA and BMI ≤ 35 kg/m2 (Group IIa) or BMI > 35 kg/m2 (Group IIb) and patients with OHS (Group III). In sitting, there was no significant difference in Rrs at any oscillometry frequency between normal subjects and those with eucapnic OSAS, although Rrs at all frequencies was higher in the eucapnic OSAS compared to normal controls when supine (#P < 0.05). In patients with OHS, significantly higher Rrs was seen at all oscillometry frequencies compared to the other three groups in both sitting and when supine (*P < 0.05). [Reprinted from Respir Physiol Neurobiol. Vol 139, Lin et al, Oral airway resistance during wakefulness in eucapnic and hypercapnic sleep apnea syndrome. pg 215‐224, 2004, with permission from Elsevier. (.)]


Figure 5. Conceptual illustration of the impact of the interapneic period on the accumulation of CO2 during sleep in patients with upper airway obstruction. The accumulation of CO2 during the hypopnea is offset during the interapnea period by sufficient ventilation over a sufficient period to eliminate the previous CO2 build up. However, if more CO2 is accumulated than can be excreted in the interapneic period, a small net rise in CO2 occurs. If this process is repeatedly frequently, a rise in PaCO2 will occur. [Reprinted with permission from Berger et al., 2002. J of Appl Physiol 93:917‐924) ().]


Figure 6. In patients with a neuromuscular and chest wall disorder, the development of nocturnal hypoventilation portends the development of awake hypercapnia within the next 12 to 24 months. These 10 patients represent a control group allocated to no NIV over a 2 year period. The light‐colored bar represents the period of time the subject remained NIV‐free. By 12 months, 70% of the group required NIV, and by 24 months only one subject did not fulfill the criteria to commence NIV. (Reproduced from Thorax, Ward et al. 60:1019‐1024, 2005 with permission from the BMJ Publishing Group Ltd) ().


Figure 7. Scatter plots (left‐hand panel) and receiver operator curves (right‐hand panel) for the predictive thresholds for Inspiratory vital capacity for the (A) onset of sleep‐disordered breathing (SDB), (B) continuous nocturnal hypoventilation and (C) diurnal respiratory failure. [Reproduced from Thorax, Ragette et al. 57:724‐728, 2002 () with permission from the BMJ Publishing Group Ltd.]


Figure 8. The “dose response” of NPPV showing the change in PaCO2 (left panel) and daytime sleepiness (ESS) (right panel) in patients with neuromuscular and restrictive thoracic disorders after 3 months of therapy. The dotted line represents the threshold below which NPPV does not have a consistent effect on PaCO2 or daytime sleepiness. Abbreviations: ESS—Epworth Sleepiness Scale; Use p.n.—use per night. [Reproduced from Thorax, Nickol et al. 60:754‐760, 2005 () with permission from the BMJ Publishing Group Ltd.]


Figure 9. Illustration of overventilation during the spontaneous mode of bilevel ventilation. Note the markedly reduced TcCO2 (right panel). Reducing CO2 below the apneic threshold will create central events which are associated with significant oxygen desaturation. Note the loss of diaphragm EMG activity along with absence of respiratory efforts on the thoracic and abdominal bands. This breathing abnormality occurs commonly at sleep onset or following arousal if excessive levels of pressure support are used in the spontaneous mode.


Figure 10. Examples of the more common asynchronies that can occur during NPPV therapy. Such events may not be apparent during wakefulness but can impact on the quality of ventilation during sleep. Panel A illustrates ineffective efforts caused by high leak. Small upward deflections can be seen in the airflow channel without a corresponding EPAP to IPAP transition on the pressure trace. Inspiratory efforts go unrewarded. Note also the generalized reduction in thoracic band movement during the leak period and a gradual fall in SpO2. The first, third, and fifth breaths on the airflow and pressure channels in Panel B show the machine has cycled twice with a very short expiratory period between the two during a single inspiratory effort (Thoracic and Abdominal bands). This can indicate pressurization is too short compared to inspiratory demand or the level of inspiratory support is too low (). Panel C illustrates autotriggering—a rapid succession of multiple machine pressurizations clearly above the patient's native respiratory rate (). This can occur if the inspiratory trigger has been set too low, especially in the presence of leak ().
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Amanda J. Piper, Brendon J. Yee. Hypoventilation Syndromes. Compr Physiol 2014, 4: 1639-1676. doi: 10.1002/cphy.c140008